少儿型'特发性'脊柱侧凸椎管内神经轴畸形发生率及相关临床特征分析

The prevalence of intraspinal neuro-axis abnormalities in juvenile patients with ' presumed idiopathic' scoliosis and the analysis of associated indicators

目的 探讨少儿型'特发性'脊柱侧凸患儿椎管内神经轴畸形发生率和类型,并分析与椎管内神经轴畸形发生相关的临床特征.方法 回顾分析2012年1月—2016年10月南京鼓楼医院脊柱外科406例少儿型'特发性'脊柱侧凸患儿的全脊髓MRI、站立位全脊柱正侧位片及相关临床资料,其中男128例、女278例,年龄(8.1 ± 1.8)岁.分析并统计经全脊髓MRI扫描后发现椎管内神经轴畸形类型和发生率,再根据有无椎管内神经轴畸形分为畸形组和非畸形组,进而比较两组间性别、年龄、主弯特征(包括Cobb角、弯型、方向、顶椎分布、节段数目)和胸椎后凸角是否存在差异.结果406患儿中有77例(18.97% )存在不同类型椎管内神经轴畸形,其中36 例患儿合并单纯 Arnold-Chiari畸形,14例患儿合并单纯脊髓空洞症,11例存在Arnold-Chiari畸形合并脊髓空洞症,4例合并脊髓栓系和脊髓纵裂症,3例合并单纯脊髓纵裂症,3例合并脊髓占位性病变,2例合并单纯脊髓栓系症,2例合并脊髓栓系、脊髓纵裂及脊髓空洞症,1例合并脊髓栓系症及脊髓占位性病变,1例合并脊髓空洞及脊髓占位性病变.畸形组中患儿男性、左胸弯和右腰弯占比分别为50.6% (39/77)、19.5% (15/77)、7.8% (6/77),高于非畸形组的27.1% (89/329)、7.0% (23/329)、0.6% (2/329),差异均有统计学意义(χ2 =17.387、4.811、15.826, P值均<0.05).结论 在少儿型'特发性'脊柱侧凸中, 18.97%的患儿存在椎管内神经轴畸形;提示少儿型'特发性'脊柱侧凸患儿存在很大必要性需行全脊髓MRI扫描以便尽早发现椎管内神经轴畸形,特别是男性、合并存在左胸弯或右腰弯患儿更应该早期行全脊髓MRI扫描.

Objective To investigate the incidence and type of intraspinal neuro-axis abnormalities in juvenile patients with'presumed idiopathic'scoliosis and to analyze the associated radiographic and clinical characteristics.Methods Four hundred and six cases of children with'esthetic'scoliosis in the spine surgery outpatient clinic of Nanjing Gulou Hospital from January 2012 to October 2016 were retrospectively analyzed.Total spinal MRI,standing full spine positive lateral radiograph and related clinical features were analyzed.There were 128 males and 278 females with an average age of(8.1±1.8)years.The type and incidence of spinal canal malformations were analyzed and counted after MRI scan.Patients were grouped into two cohorts according to the presence of neural axis abnormalities.Radiographic parameters including curve magnitude,direction of main curve,curve pattern,location of apex,degree of thoracic kyphosis,and span of curve were recorded and compared between the two groups.Results Of the 406 children,77 patients(18.7%)had different types of spinal cord malformations:of which 36 patients had isolated Arnold-Chiari malformation,14 patients had isolated syringomyelia,11 patients had Arnold-Chiari malformation combined with syringomyelia,4 patients had tethered cord combined with diastematomyelia,3 patients had spinal cord fissure,3 patients had spinal cord occupying lesions,2 patients had isolated tethered cord disease,2 patients had syringomyelia and tethered cord combined with diastematomyelia,one patient had tethered cord combined with spinal core occupying lesion,and one patient had syringomyelia combined with spinal cord occupying lesion.Compared with the non-abnormal group,the male 50.6%(39/77),left chest 19.5%(15/77),and right lumbar flexion ratios 7.8%(6/77)were higher in the deformed group 27.1(89/329),7.0%(23/329),0.6%(2/329)(χ2=17.387,4.811,15.826,all P values<0.05).Conclusions The incidence of neural axis abnormalities in patients with the presumed JIS is 18.7%.There is a great need for children with JIS to have a full spinal MRI scan in order to detect spinal canal malformations as early as possible,especially in males with left thoracic curve or right lumbar curve.